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Díaz, Roberto; Aparicio, Jorge; Pous, Salvador; Dolz, Jose F.; Calderero, V.

doi:10.1023/a:1024700831418

Cited by 21 publications

(12 citation statements)

References 13 publications

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“…As a result of the limited success of pre-operative localization, metastatic disease may mimic a primary tumour as the size of a duodenal primary is not strongly linked to its malignant potential. Of these 27 cases of PHG, 23 including the present case described a reasonable approach to pre-operative localization [4,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,25,26,27]. Of the four remaining cases, one was diagnosed at autopsy (#18), the second was diagnosed after removal of a presumed malignant liver tumour (#6), the third underwent an apparently blind exploration following the biochemical diagnosis (#23) and the fourth only described a preoperative CT scan (#15) [3,6,23,24].…”

Section: Discussionmentioning

confidence: 99%

“…Only seven cases out of 26 reported follow-up of more than 5 years [6,14,18,20,27]. Recurrence was seen in four patients [8,14,18,20]. Two of these were local recurrences in the liver (#8), one of which reported multiple liver lesions (#22) [14,18].…”

Section: Discussionmentioning

confidence: 99%

“…Two of these were local recurrences in the liver (#8), one of which reported multiple liver lesions (#22) [14,18]. One recurrence was seen in the lymph node along the lesser curvature of the stomach (#13) and the fourth was a biochemical recurrence (#12) [8,20]. A slight elevation in FSG following liver resection was reported in case #14.…”

Section: Discussionmentioning

confidence: 99%

“…It is likely that the liver lesion was metastatic disease from an occult duodenal primary. In addition, patients #4, #13, #14, #17, #21 were below the age of 13 at diagnosis increasing the index of suspicion for hereditary disease [8,11,13,15,26]. Long-term follow-up and genetic testing would be required on these patients before one could confidently establish the diagnosis of PHG.…”

Section: Discussionmentioning

confidence: 99%

“…To date only 26 cases of apparently PHG have been reported in the English language literature (Table 1) [3,4,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. All of these cases were confirmed histopathologically and 23 had documented preoperative biochemical evidence of ZES.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis

Harvey

Pasieka

Albisher

et al. 2012

Cancers

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The majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermore, gastrinomas are typically slow-growing thus a missed, occult primary tumour may not become evident for many years. The diagnosis of PHG is therefore dependent on a careful search for a primary and long-term biochemical follow-up following curative hepatic resection. We report a case of a 7 cm PHG in a 48 year old man with ZES. Preoperatively, both a basal and stimulated gastrin levels were elevated. Surgical exploration including intraoperative ultrasound and duodenotomy, failed to reveal a primary. Patient underwent a right hepatectomy. Yearly, gastrin and secretin stimulation tests remain normal 6 years following surgery. He remains symptom free off all medication. An additional 26 cases of PHG were found. Including this case, 21 had at least 1 year follow-up, however only eight had greater than 5 years (median 24 months). Post-op gastrin levels were reported in 25, however provocative testing was done in only 10. Persistence and recurrence occurred in one and four, respectively. PHG causing ZES is extremely rare. Although the current literature claims to include 26 additional cases of PHG, without a thorough search for the primary and long-term follow-up data including provocative testing, this diagnosis remains a challenge.

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